This gene encodes a member of the ubiquitin-specific
processing (UBP) family of proteases that is a deubiquitinating
enzyme (DUB) with His and Cys domains. This protein is located in
the cytoplasm and cleaves the ubiquitin moiety from ubiquitin-fused
precursors and ubiquitinylated proteins. Mice with a mutation that
results in reduced expression of the ortholog of this protein are
retarded for growth, develop severe tremors by 2 to 3 weeks of age
followed by hindlimb paralysis and death by 6 to 10 weeks of age.
Alternate transcriptional splice variants, encoding different
isoforms, have been characterized.
